Some years ago I read about a horrible disease named Kuru. It was listed in The Guinness Book of World Records as the most rare disease in the world. A little investigation, however, showed that Kuru was not all that rare.
Called “laughing sickness”, Kuru is a brain wasting disease that is transmitted by curious means: you get it from eating the brain tissue of other infected humans. Most of us have little reason to worry.
This is a good thing because Kuru is a terrible disease. The epidemic peaked in the 1960’s, killing over 1,100 people in the South Fore region of New Guinea. The tribes of this area practiced mortuary cannibalism — they ate their dead, and certain portions of the anatomy were ritually prepared for consumption in religious ceremonies. The brain tissue, in particular, was prized for this purpose. Kuru was thought to be a genetic disease, so there was no fear of eating those who died from it. In fact, Kuru victims were particularly prized for consumption, due to the quick death and the thick layer of fat left on the bodies [Prion Diseases; McGrath].
It is no wonder that for decades scientists did not attribute the transmission of the disease to the cannibalistic practices of the natives. Kuru has an incubation period of two to 23 years, and kills in about 3 months. During this time, the victim progresses through three stages of degeneration:
The ambulant stage includes unsteadiness of stance, gait, voice, hands, and eyes; deterioration of speech; tremor; shivering; in-coordination in lower extremities that moves slowly upward; and slurred speech.
In the sedentary stage, the patient can no longer walk without support, suffers more severe tremors and ataxia (loss of coordination of the muscles), shock-like muscle jerks, emotional labiality, outbursts of laughter, depression, and mental slowing.
In the terminal stage – which is marked by the patient’s inability to sit up without support – there is more severe loss of muscle coordination, tremor, and slurring of speech; urinary and fecal incontinence; difficulty swallowing; and deep ulcerations appear.
——-[Paraphrased from McGrath]
These symptoms may sound familiar. This is because Kuru is what has been termed a Prion Disease — a medical curiosity where a disease is passed from one victim to another by a rogue protein (a prion) that has no DNA, RNA, or any other known route of transmission [Access Excellence]. Another Prion disease is Creutzfeldt-Jakob disease (CJD), a variant of which (vCJD) is the cause of Mad Cow [aka, bovine spongiform encephalopathy of BSE].
North American citizens are unlikely to ever contract Kuru, but this is not so for CJD. There are other known Prion Diseases, including Scrapie in sheep, and Chronic Wasting Disease (CWD) that has infected many deer and elk in North America. These diseases are often called spongiform encephalies, because they leave the brain tissue full of holes, and all are fatal. The new variant of BSE (mad Cow) kills in about 18 months, while the older one was fatal in 6 [Why Files].
Most North Americans don’t worry much about Mad Cow. We are told that no cow on our continent has been found with the disease, and that the deer and elk form of the disease that is cropping up here is not transmittable to humans. However, there mad cows in Britain for years, which were routinely fed to humans because the government was assured that the disease could not cross the species boundary. The first few cases of cross-species transmission were ignored, as CJD is known to occur spontaneously in a very small percentage of the population.
In reality, eating these cows was spreading mad cow in the very same way that the spread of Kuru began. Many New Guinea tribes practiced mortuary cannibalism, but the disease seemed to only effect a few. It is believed that one person may have acquired the disease through a natural genetic mutation, and that the practice of consuming the brain tissue is what started it spreading throughout the tribal group. In a similar fashion it is believed that cows first contracted Mad Cow through ingesting feed that was supplemented with meat from sheep infected with Scrapie, which was known to exist in sheep for over 200 years [Access Excellence]. Still, the North American governments insist that we do not have to be afraid of contracting any form of Prion disease from infected deer or elk.
To support this theory, the government points out that no one has gotten sick from eating sick deer. This is true, however, this may not mean anything at all. You see, Mad Cow is now thought to have an incubation period of as long as 30 years [Mercola]. There is evidence that when the diseases cross the species boundary, they are slow moving at first, and then progress more quickly through subsequent generations as the protein adapts to the new species.
“Aiken, for example, passed a mink [Prion Disease] … through hamsters, and found that the agent acted faster in successive generations. The first hamsters [infected] generally outlived the disease, which took hundreds of days to incubate. When brain material from those hamsters was injected into other hamsters, the disease struck faster. By the third generation, there was a ‘tremendous shortening — it has adapted to the new species.'” (Why Files)
This means that those who have ingested the meat of animals carrying the prions in North America may not show any symptoms for years, but that progression will speed up significantly over time just as it has in Britain. This also means that the 80 deaths attributed to Mad Cow so far in Britain are just the tip of the iceberg. In fact, many scientists are projecting that once all of those already infected succumb to the disease, that the death toll with be somewhere between 10,000 and 100,000 [Mercola; Why Files].
This week, a Saskatchewan man was the first Canadian victim of Mad Cow. He is said to have contracted the disease during a stay in Great Britain in the late eighties, but there is no evidence to support this theory. Still, Antonio Giulivi, director of Health Canada’s Acquired Infections branch believes that we will likely have more Canadian cases from people who travelled to Britain during that time [Canadian Press, 2002].
Giulivi, however, also tries to reassure people with the ridiculous palliative statement: “The risk of coming down with this disease is much lower than a person smoking and getting cancer.”
Given that an estimated 66,200 [Stats Canada] Canadians will die of cancer this year, and that 30% of these are attributed to smoking, then about 19,860 people will die from smoking related cancer this year — and this only takes into account mortality — many more smokers will get cancer and survive. Dr. Giulivi’s reassurance, then, is laughable. Perhaps if he were more certain, he’d say that we had a greater chance of being hit by lightening, or something equally unlikely.
Despite all this, most people in North America have chosen to remain unconcerned, like Nadine Vosper of West Vancouver who visits relatives in Britain and eats British meat. She brushes off the concern, stating: “If you worry about every little thing you can’t live [Canadian Press].” Perhaps, but is this a little thing? Oddly, many people on both sides of the ocean have taken an almost defiant approach to the scare, declaring that they have eaten beef all of their lives, and will never stop. What, exactly, are these people trying to defy? Death itself? Certainly not that infectious little prion, for it cares little for stoicism.
It is alarming, then, that we are doing so little to prevent the disease. Britain is still killing cattle for the dinner table, despite knowing that the disease is undetectable in the cows in the early stages, and that it cannot be killed by any process that can kill bacterium or viruses. Certainly, if a brand of hair dye or cold medicine were found to be responsible for this many deaths, it would be pulled off the shelves, but there has been no ban on beef because they are afraid of angering consumers. Unfortunately, this may be the only way to stop the disease.
When the tribes of New Guinea stopped cannibalizing their dead (a practice that had great historical and religious significance), Kuru became a part of medical history. Mad Cow may have disappeared if beef production had been stopped for a few years to clean up the herds, but because these steps were not taken the disease is spreading to more animals and all of our meat is threatened. It seems that most people would rather take that risk than to live without steaks and hamburgers for a while.
It may be a self-defeating risk, because if the disease cannot be stopped we may never be able to eat beef again and other meats may follow. The American government has attempted to curb the disease by making it illegal to feed cattle feed products that contains cow parts, but they were slow in doing so, and they have not been very effective in enforcing the ban. Just last year “a blunder in a Texas feed mill showed that regulations on feed are not foolproof. Despite quick corrective action, regulators and industry officials, to say nothing of food safety activists, were upset that the mill supplied a Texas feedlot with feed containing parts of slaughtered cattle — the exact recipe that distributed mad cow across the United Kingdom more than 10 years ago [Why Files].”
This is especially alarming in light of a British Report [The BSE Inquiry] which “indicated that a chunk of prion-laced meat as small as a peppercorn could infect a cow — and that thousands of British cows were infected simply because cow feed was contaminated by pig feed while being handled in grain mills [Why Files].” Nevertheless, many are confident that current protection measures are adequate; although the Centers for Disease Control and Prevention budget for detecting the onset of BSE is only $100,000 [Why Files]. There is already some evidence that the deer and elk variant of Chronic Wasting Disease may be able to infect humans just as easily as the cattle variant [Raymond, et al.], but no bans on hunting are in place. They must be mad…
Access Excellence. Mad Cow Disease: The BSE Epidemic in Great Britain. Access Excellence @ The National Health Museum. Online at: http://www.accessexcellence.org/WN/NM/madcow96.html
BSE Inquiry, The. The Inquiry into BSE and variant CJD in the United Kingdom. Online at: http://126.96.36.199/index.htm
Canadian Press, 2002: Friday, Aug 09. Health officials remain vigilant for more cases of mad cow illness in people. Online at: http://www2.alberta.com/news/health/fs.cfm?source_id=CP&id=1160894
Mad Cow.com. Online at: http://organicconsumers.org/madcow.htm
McGrath: Kuru: The Dynamics of a Prion Disease. Physical Anthropology Department at the University of Alabama. Available online at: http://www.as.ua.edu/ant/bindon/ant570/Papers/McGrath/McGrath.htm
See additional cited references in bibliography for this article.
Mercola. Online at: http://www.mercola.com/beef/incubation.htm
Prion Diseases: http://www-micro.msb.le.ac.uk/335/Prions.html
Raymond, et al.: Evidence of a molecular barrier limiting susceptibility of humans, cattle and sheep to chronic wasting disease. Raymond GJ, Bossers A, Raymond LD, O’Rourke KI, McHolland LE, Bryant III PK, Miller MW, Williams ES, Smits M, Caughey B. The EMBO Journal, Vol. 19, No. 17 pp. 4425-4430, 2000
Statistics Canada: http://www.cancer.ca/files/stats2002_e.pdf
Why Files. Cow Madness: A Beef With Beef. Why Files. Online at: http://whyfiles.org/012mad_cow/
Tamra lives in Calgary with her husband and two cats. A fulltime AU student, she splits her free time between her duties as an AUSU councillor, writing her first novel, and editing written work by other students and friends.